Mitochondrial BTD hydrolyses BCTN

Stable Identifier
Reaction [transition]
Homo sapiens
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Human biotinidase (BTD, EC (Cole et al. 1994) catalyzes the hydrolysis of biocytin (BCTN, aka biotinyl-lysine), a product of biotin dependent carboxylase degradation, to biotin (Btn) and lysine. As a result, Btn is again available to be used in the biotinylation of apo-carboxylases in the mitochondrion. BTD is both secreted from various cells and localised in the mitochondria (Wolf & Jensen 2005). BTD deficiency, an autosomal recessive disorder, results in a secondary Btn deficiency that leads to juvenile onset multiple carboxylase deficiency (MIM:253260) (Wolf et al. 1983).

Literature References
PubMed ID Title Journal Year
16150625 Evolutionary conservation of biotinidase: implications for the enzyme's structure and subcellular localization

Wolf, B, Jensen, K

Mol. Genet. Metab. 2005
7509806 Human serum biotinidase. cDNA cloning, sequence, and characterization

Hymes, J, Wolf, B, Denson, T, Cole, H, Spence, JE, Lockyer, JM, Reynolds, TR, Buck, GA

J. Biol. Chem. 1994
6883721 Biotinidase deficiency: the enzymatic defect in late-onset multiple carboxylase deficiency

Wolf, B, Allen, RJ, Goodman, SI, Grier, RE, Kien, CL

Clin. Chim. Acta 1983
Catalyst Activity

biotinidase activity of BTD [mitochondrial matrix]

Orthologous Events
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